[Nesidioblastosis. Apropos of 12 new cases].
Two new cases of diffuse hyperplasia of the pancreas are reported. This infrequent condition is caused by intermittent and variable insulin hypersecretion. The hyperinsulinism is responsible for severe, lasting and intractable hypoglycemia that causes seizures and mental retardation. Onset usually occurs in the neonatal period. The diagnosis of hyperinsulinism rests on four criteria: the presence of increased insulin levels in the face of hypoglycemia, the low urinary excretion of ketone bodies during hypoglycemic episodes, the need for more than 15/mg/kg/min glucose to maintain the serum glucose level above 2 mmol/l, and a positive response to glucagon. The topographic diagnosis is often disappointing. Medical treatment of the hypoglycemia with diazoxide is a transient measure. Subtotal pancreatectomy is indispensable. Postoperative results are variable. Insulin deficiency diabetes mellitus is common and unusual in that insulin induces an exaggerated response. Recovery can be observed. If hypoglycemia recurs, diazoxide is often effective.
Samoud
A
A
Service de Pédiatrie, Hôpital la Rabta, TUNIS, Tunisie.
Dey
D
D
Brauner
R
R
Doagi
M
M
Kallel
H
H
Boubaker
S
S
Osman
R
R
Ben Dridi
M F
MF
D005260:Female; D006801:Humans; D006946:Hyperinsulinism; D007003:Hypoglycemia; D007223:Infant; D008297:Male; D010182:Pancreatic Diseases / Q000503:physiopathology
La nésidioblastose. A propos de deux nouvelles observations.